| Title |
Spinal Cord Damage in Machado-Joseph Disease
|
|---|---|
| Published in |
The Cerebellum, November 2014
|
| DOI | 10.1007/s12311-014-0619-7 |
| Pubmed ID | |
| Authors |
Camila N. Fahl, Lucas Melo T. Branco, Felipe P. G. Bergo, Anelyssa D’Abreu, Iscia Lopes-Cendes, Marcondes C. França |
| Abstract |
Machado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in the disease and its clinical relevance. To evaluate the spinal cord damage in SCA3 through quantitative analysis of MRI scans. A group of 48 patients with SCA3 and 48 age and gender-matched healthy controls underwent MRI on a 3T scanner. We used T1-weighted 3D images to estimate the cervical spinal cord area (CA) and eccentricity (CE) at three C2/C3 levels based on a semi-automatic image segmentation protocol. The scale for assessment and rating of ataxia (SARA) was employed to quantify disease severity. The two groups-SCA3 and controls-were significantly different regarding CA (49.5 ± 7.3 vs 67.2 ± 6.3 mm(2), p < 0.001) and CE values (0.79 ± 0.06 vs 0.75 ± 0.05, p = 0.005). In addition, CA presented a significant correlation with SARA scores in the patient group (p = 0.010). CE was not associated with SARA scores (p = 0.857). In the multiple variable regression, we found that disease duration was the only variable associated with CA (coefficient = -0.629, p = 0.025). SCA3 is characterized by cervical cord atrophy and antero-posterior flattening. In addition, the spinal cord areas did correlate with disease severity. This suggests that quantitative analyses of the spinal cord MRI might be a useful biomarker in SCA3. |
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 1 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Science communicators (journalists, bloggers, editors) | 1 | 100% |
Mendeley readers
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Germany | 1 | 3% |
| Unknown | 36 | 97% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 9 | 24% |
| Student > Master | 7 | 19% |
| Student > Bachelor | 3 | 8% |
| Student > Postgraduate | 3 | 8% |
| Professor | 1 | 3% |
| Other | 2 | 5% |
| Unknown | 12 | 32% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 7 | 19% |
| Neuroscience | 7 | 19% |
| Agricultural and Biological Sciences | 3 | 8% |
| Biochemistry, Genetics and Molecular Biology | 2 | 5% |
| Psychology | 1 | 3% |
| Other | 3 | 8% |
| Unknown | 14 | 38% |
Attention Score in Context
This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 01 December 2014.
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#16,272,032
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Outputs of similar age from The Cerebellum
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